Fibrinogen New Orleans: hereditary dysfibrinogenemia with an A alpha chain abnormality.
An abnormal fibrinogen (Fibrinogen New Orleans, or FNO) has been found in a 30 year-old woman, her mother and daughter, but not her father. The propositus suffered mild bleeding, but not thrombo-embolism or abnormal wound healing. Plasma and purified fibrinogen from the propositus caused a prolongation in the clotting time of normal plasma. FNO had increased anodal migration when studied by immunoelectrophoresis. We have been able to follow the release of fibrinopeptides A and B using sensitive SDS-PAGE. In FNO, release of the A peptide was markedly delayed, whereas the cleavage of B peptide was much less delayed, compared to that of A peptide.
D000328:Adult; D000721:Ancrod / Q000494:pharmacology; D000941:Antigens; D001778:Blood Coagulation Disorders / Q000097:blood / Q000235:genetics*; D001780:Blood Coagulation Tests; D003432:Cross-Linking Reagents / Q000494:pharmacology; D005260:Female; D005340:Fibrinogen / Q000032:analysis / Q000235:genetics* / Q000276:immunology / Q000378:metabolism; D015241:Fibrinogens, Abnormal; D005342:Fibrinolysis; D005344:Fibrinopeptide A / Q000235:genetics* / Q000378:metabolism; D006801:Humans; D005779:Immunodiffusion; D007123:Immunoelectrophoresis, Two-Dimensional; D008970:Molecular Weight; D013917:Thrombin / Q000494:pharmacology
D000941:Antigens; D003432:Cross-Linking Reagents; D015241:Fibrinogens, Abnormal; C033919:fibrinogen New Orleans; D005344:Fibrinopeptide A; D005340:Fibrinogen; D000721:Ancrod; D013917:Thrombin